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巨细胞动脉周围炎

Giant cell arteritis

定义 英文原文(暂无中文)

A rare large vessel vasculitis (LVV) characterized by vasculitis predominantly involving the arteries originating from the aortic arch and the extracranial branches of the carotid arteries. Clinical manifestations are variable, the predominant cranial phenotype is characterized by headache, jaw claudication, scalp tenderness and visual symptoms and the predominant LVV type by constitutional symptoms, polymyalgia rheumatica and occasionally limb ischemia. Overlaps between these two phenotypes are common.

别名

颞动脉炎

基本事实

遗传方式
多基因/多因素
发病年龄
成年期
患病率
1-9 / 100 000(Germany)

相关基因 4

基因名称关联类型
HLA-Bmajor histocompatibility complex, class I, BMajor susceptibility factor in
HLA-DRB1major histocompatibility complex, class II, DR beta 1Major susceptibility factor in
PTPN22protein tyrosine phosphatase non-receptor type 22Major susceptibility factor in
P4HA2prolyl 4-hydroxylase subunit alpha 2Major susceptibility factor in

临床表型 73

极常见 99–80%10

  • 厌食症 HP:0002039
  • 脑缺血 HP:0002637
  • C-反应蛋白水平升高 HP:0011227
  • 疲乏 HP:0012378
  • 发热 HP:0001945
  • 头痛 HP:0002315
  • 咀嚼功能受损 HP:0005216
  • 关节僵硬 HP:0001387
  • 血管炎 HP:0002633
  • 体重减轻 HP:0001824

常见 79–30%18

  • 全身血压异常 HP:0030972
  • 脱发 HP:0001596
  • 贫血 HP:0001903
  • 关节炎 HP:0001369
  • 视力模糊 HP:0000622
  • 颈动脉痛 HP:6000944
  • 抑郁 HP:0000716
  • 循环钙卫蛋白水平升高 HP:6000502
  • 循环骨调素水平升高 HP:0033123
  • 红细胞沉降速度升高 HP:0003565
  • 高纤维蛋白原血症 HP:0011899
  • α-球蛋白增加 HP:0005413
  • 循环白细胞介素6水平升高 HP:0030783
  • 颌跛行 HP:0030164
  • 全身乏力不适 HP:0033834
  • 眼肌瘫痪 HP:0000597
  • 多发性关节炎 HP:0005764
  • 视觉障碍 HP:0000505

偶见 29–5%45

  • 腹主动脉瘤 HP:0005112
  • 腹痛 HP:0002027
  • 胸膜异常 HP:0002103
  • 血小板异常 HP:0001872
  • 颞浅动脉搏动消失 HP:6000786
  • 一时性黑蒙 HP:0100576
  • 主动脉夹层 HP:0002647
  • 心律失常 HP:0011675
  • 动脉血栓形成 HP:0004420
  • 关节疼痛 HP:0002829
  • 共济失调 HP:0001251
  • 传导性听力受损 HP:0000405
  • 咳嗽 HP:0012735
  • 尿崩症 HP:0000873
  • 复视 HP:0000651
  • 右室双出口伴肺动脉下室间隔缺损无肺动脉狭窄 HP:0011658
  • 构音障碍 HP:0001260
  • 鼻衄 HP:0000421
  • 坏疽 HP:0100758
  • 胃肠道梗塞 HP:0005244
  • 舌炎 HP:0000206
  • 听力受损 HP:0000365
  • 血尿 HP:0000790
  • 肝功能衰竭 HP:0001399
  • 多汗症 HP:0000975
  • 纵膈淋巴结病 HP:0100721
  • 脑膜炎 HP:0001287
  • 肌无力 HP:0001324
  • 肌痛 HP:0003326
  • 干咳 HP:0031246
  • 眼球震颤 HP:0000639
  • 视神经萎缩 HP:0000648
  • 感觉异常 HP:0003401
  • 心包炎 HP:0001701
  • 周围神经病 HP:0009830
  • 上睑下垂 HP:0000508
  • 复发性咽炎 HP:0100776
  • 肾功能不全 HP:0000083
  • 头皮压痛 HP:0100809
  • 皮肤溃疡 HP:0200042
  • 心脏性猝死 HP:0001645
  • 眩晕 HP:0002321
  • 视野缺损 HP:0001123
  • 幻视 HP:0002367
  • 视力丧失 HP:0000572

近两年的全球研究 2,086L2

2024/10 起在 Europe PMC 检索所得,按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务,尚未引入。

  • 2026-10开放获取
    Study on the implementation of the 2015 EULAR/ACR recommendations for polymyalgia rheumatica in clinical practice
    RMD open · DOI · Europe PMC
  • 2026-10开放获取
    Reduced humoral response to recombinant zoster vaccine in patients with SLE: a prospective cohort study across immune-mediated inflammatory diseases
    Lupus science & medicine · DOI · Europe PMC
  • 2026-10病例报告开放获取
    Drug-Induced Liver Injury During Treatment With Tocilizumab: A Case Report of an Atypical Pattern Lesion
    Pharmacology research & perspectives · DOI · Europe PMC
  • 2026-10
    Diagnostic accuracy of musculoskeletal ultrasound in suspected polymyalgia rheumatica: a multicentre, cohort study
    The Lancet. Rheumatology · DOI · Europe PMC
  • 2026-10荟萃分析系统综述综述
    Follow-Up 18F-Fluorodeoxyglucose Positron Emission Tomography in Treated Patients With Giant Cell Arteritis: A Systematic Review and Metaanalysis
    The Journal of rheumatology · DOI · Europe PMC
  • 2026-10
    Structured Vascular Segment Analysis for Giant Cell Arteritis: A Proposed Scoring System for CTA-Based Diagnosis
    AJNR. American journal of neuroradiology · DOI · Europe PMC
  • 2026-09
    Paracentral acute middle maculopathy in giant cell arteritis versus non-arteritic anterior ischaemic optic neuropathy
    The British journal of ophthalmology · DOI · Europe PMC
  • 2026-09综述开放获取
    The Immune Checkpoint Inhibitors Journey: From Early Promise to Lasting Impact
    Journal of immunotherapy and precision oncology · DOI · Europe PMC
  • 2026-09
    The amalric sign: A clue to giant cell arteritis
    European journal of ophthalmology · DOI · Europe PMC
  • 2026-09
    International practice patterns and beliefs concerning intravenous methylprednisolone and low-dose aspirin in giant cell arteritis
    Eye (London, England) · DOI · Europe PMC
  • 2026-09开放获取
    Comparison of four clinical risk prediction models for the diagnosis of giant cell arteritis: results from the ongoing PREDICT-GCA study
    RMD open · DOI · Europe PMC
  • 2026-09
    Impact of corticosteroid use on quality of life of people over 65 years of age with giant cell arteritis and/or polymyalgia rheumatica
    Geriatric nursing (New York, N.Y.) · DOI · Europe PMC
  • 2026-09
    [Management of emergencies in rheumatology]
    Zeitschrift fur Rheumatologie · DOI · Europe PMC
  • 2026-09
    Real-World Effectiveness and Safety of Secukinumab in Giant Cell Arteritis: An Italian Multicenter Cohort Study
    Arthritis care & research · DOI · Europe PMC
  • 2026-09综述开放获取
    Anti-obesity medications impacting the eye: An emerging concern - a narrative review
    World journal of methodology · DOI · Europe PMC
  • 2026-09
    Infection Risk Associated with Steroid-Sparing Therapies in GCA, PMR, and ANCA-Associated Vasculitis: A Systematic Review
    Joint bone spine · DOI · Europe PMC
  • 2026-09病例报告开放获取
    Silicoanthracosis: A melanoma mimicker
    Radiology case reports
  • 2026-09
    Retraction: Association Between Baseline Aortic 18F-Fluorodeoxyglucose Uptake and Subsequent Aortic Dilatation in Giant Cell Arteritis: A Four-Year Post Hoc Analysis
    Cureus · DOI · Europe PMC
  • 2026-09综述开放获取
    Bridging Autoimmunity and Hyperinflammation: Shared Immunological Pathways Among Autoimmune/Inflammatory Syndrome Induced by Adjuvants, Systemic Lupus Erythematosus, and Macrophage Activation Syndrome
    Biomedicines · DOI · Europe PMC
  • 2026-09
    Orbital Inflammation can be visualized by black blood MRI in isolated polymyalgia rheumatica
    Rheumatology (Oxford, England) · DOI · Europe PMC

境外已获批用于本病的药物 3L2

欧盟 2 项、美国 1 项。同一药物在两地各批一次的,会分别列出。

「境外已获批」不等于「在中国能用」。中间隔着进口注册、临床急需境外新药通道、海南博鳌乐城国际医疗旅游先行区等几条路径,各有各的条件与费用。这一节能确定地告诉你的只有一件事:这个病在世界范围内已经有获得批准的药物,它叫什么名字。拿这个名字去问主治医生,是下一步最省力的做法。

药名一律保留英文原文,不作翻译——中国的药品通用名与英文名的音译经常不一致,译错会让人去找一个不存在的药。

尚未获批的在研药物(1 项)

这些药已被欧盟或美国的监管机构认定为罕见病用药(英文 orphan drug designation,中文行业里通称「孤儿药资格」——「孤儿」说的是这类药市场太小、没有厂商愿意认领,不是在说病人)。但这只是一种监管身份:意味着监管机构给予研发上的激励,不代表这个药已被证明有效,也不代表将来一定能上市,绝大多数最终不会成药。列在这里是为了看清有哪些方向正在被尝试。

  • Abatacept美国2017-02-22
    Treatment of giant cell arteritis
    官方记录

数据来自欧洲药品管理局(EMA)的药品与罕见病用药资格公开导出表,以及美国 FDA 的罕见病用药资格数据库(Orphan Drug Product Designation Database)。两边口径不同:欧盟一侧取的是当前状态仍为「已授权」的药品;美国一侧记录的是「曾获批准」这一事实,FDA 的公开表不追踪药物此后是否退市(例如 Relyvrio 于 2024 年撤市,表中仍记为已获批)。请以官方记录页为准。

在中国开展的临床试验 4L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实,并与主治医生商量。

登记为可入组 1

  • 尚未开始招募NCT06766552
    A Registry Study Assessing PRO, Dosing Patterns, and Safety of Vunakizumab in Patients With General Rheumatic Diseases.
    观察性 · 2025/01/30Second Affiliated Hospital, Zhejiang University, School of Medicine
    中国研究中心 1 个:Hangzhou
其他状态的试验(3 项)
  • 状态未知NCT01066208
    American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis
    观察性 · 2011/01University of Oxford
    中国研究中心 1 个:Beijing
  • 已完成NCT04799262
    Tofacitinib as a GC Sparing Agent for Polymyalgia Rheumatica
    II 期 · 干预性 · 2021/01/01RenJi Hospital
    中国研究中心 1 个:Shanghai
  • 已完成NCT06172361
    Induction and Tapering Therapy With Tofacitinib and Glucocorticoid in Patients With Polymyalgia Rheumatica
    III 期 · 干预性 · 2024/01/20Zhejiang University
    中国研究中心 1 个:Hangzhou

中国境外的在招试验 55L2

这些试验在中国没有研究中心,通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处:看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生,或据此进一步查该药是否已在境外获批、是否有拓展性用药(expanded access)通道。

法国14美国10加拿大7德国7丹麦6英国4瑞士3瑞典1意大利1Armenia1土耳其1

CT.gov 报告命中 55 项,此处取回并展示最近的 15 项。

  • 尚未开始招募NCT07269938
    Mapping B-cell Biology Across the Cardiovascular Territories of Giant Cell Arteritis: Towards a New Therapeutic Approach (RituxiMAP GCA)
    观察性 · 2026/10University of Edinburgh
    英国
  • 尚未开始招募NCT07737470
    Inflammatory Disease Biobank for Immunophenotyping and Cardiovascular Research
    观察性 · 2026/09/01Assistance Publique - Hôpitaux de Paris
  • 尚未开始招募NCT07748195
    DNA Methylation in PMR
    观察性 · 2026/08/31Dartmouth-Hitchcock Medical Center
  • 尚未开始招募NCT07749547
    Using Personalized Rehabilitation to Reduce Disease and Increase Activity of Patients With Polymyalgia Rheumatica
    不适用 · 干预性 · 2026/08/15Washington University School of Medicine
  • 尚未开始招募NCT07132164
    Justification of the Initial Diagnosis and Evaluation of the Overall Evolution of a Cohort of Recent Polymyalgia Rheumatica (JADORE)
    观察性 · 2026/07/01University Hospital, Brest
    法国
  • 尚未开始招募NCT07628075
    Long-Axial Field of View (LAFOV) [18F]FDG PET/CT Imaging in Large Vessel Vasculitis (LVV): Protocol Optimisation Study.
    不适用 · 干预性 · 2026/07/01Imperial College London
    英国
  • 尚未开始招募NCT06833411
    Treatment for Giant Cell Arteritis With Tocilizumab and 8 as Compared to 26 Weeks of Prednisone
    III 期 · 干预性 · 2026/07Insel Gruppe AG, University Hospital Bern
    瑞士
  • 尚未开始招募NCT07720804
    Kinetics of C-Reactive Protein in Giant Cell Arteritis Following Glucocorticoid Initiation: A Pilot Study (GIDEON)
    不适用 · 干预性 · 2026/06/22University Hospital, Limoges
  • 尚未开始招募NCT06957002
    Bosentan in the Treatment of Giant Cell Arteritis
    II 期 · 干预性 · 2026/06Assistance Publique - Hôpitaux de Paris
    法国
  • 招募中NCT07286214
    Sarilumab Efficacy and Safety in Adults With Early Polymyalgia Rheumatica
    IV 期 · 干预性 · 2026/05/12Sanofi
    加拿大、美国
  • 招募中NCT07552636
    Disease Activity Monitoring in Patients With Giant Cell Arteritis Study
    观察性 · 2026/05/06University of Aarhus
    丹麦
  • 尚未开始招募NCT07508514
    Early Temporal Dynamics of Optic Nerve Sheath Diameter After Therapy in GCA
    观察性 · 2026/05/01Centre Integre Universitaire de Sante et Services Sociaux du Nord de l'ile de Montreal
    加拿大
  • 尚未开始招募NCT07354906
    Upadacitinib in Giant Cell Arteritis (GCA) With Active Large-vessel Involvement.
    观察性 · 2026/03/01Groupe français d'étude des Maladies Inflammatoires de loeil
    法国
  • 招募中NCT07246577
    The VGR GCA Cohort: Ultrasound, Biopsy and Biomarkers - Novel Methods for Diagnosis, Monitoring and Prognosis in Giant Cell Arteritis.
    观察性 · 2026/03/01Vastra Gotaland Region
    瑞典
  • 招募中NCT07060274
    Halo Sign Vanishing Time After Steroids Outbreak in GCA Patients
    不适用 · 干预性 · 2026/02/27Centre Hospitalier le Mans
    法国

外部标识与链接

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)